Tag Archives: CHD
It’s Father’s Day. Where’s the Baby Book?

It’s Father’s Day. Where’s the Baby Book?

I remember our daughter Eve’s first haircut.  It happened on Saturday night, at 2 AM.  She was 9 days old.

Paul and I were in the usual half-sleep that comes with having a child in an ICU.  We were down the hall from the PICU at the University of Minnesota Children’s hospital, and had just heard the dreaded “code blue” come over the intercom system.  This time was different.  About 2 minutes later, my cell phone rang.  It was the ICU attending physician asking us to come down.  I flew out of our makeshift floor-bed and RAN like hell the 50 or so yards to where Eve was.  “Is it Eve??”  No, calm down, they said.  Apparently all her tiny veins were blown out from days of fluids and meds being pumped into the body of 5.5 pound newborn…the only place left to place the IV was in her head.  They needed to shave some of her beautiful dark hair

Eve Saarinen, with Elle and Jack, 10 days old, U of M Children's PICU

- and didn’t want us to freak out when we came in the morning.  They saved her locks of hair for us.  We still have them to this day.

What we don’t have is the proverbial Baby Book to put them in.  There’s nothing on paper historically documenting Eve’s first coo.  Her first smile. Her first babble, first tooth or first roll-over.  This, I suspect, is a sad reality for too many of the families whose babies face critical illnesses. There are simply more important things to focus on when your kid is fighting to survive. I just told my mom this morning that I cannot recall being at Eve’s baptism. I know we had one…it was at our church even.  But it wasn’t long before we were prepping to go to Boston Children’s Hospital for her heart surgeries and our brains must have been mush. Gratefully we live in the era of cell phone cameras, or we probably wouldn’t have any photos of Eve. Frankly, it’s a little tough to be in “photo shoot” mode while your baby is hooked up to a dozen lines and monitors.  While these images may be hard for others to look at, we treasure those grainy pictures.  She was so serene and lovely – yet tough as nails.

Eve Saarinen, April 2009

We also live in the era of Facebook, Twitter and blogs. Eve is fortunate to have a daddy who qualifies as an “early adopter” of these methods of communication and historical documentation. Random fact: Eve’s older sister Elle’s birth was hailed by the Sydney Morning Herald as the first live-tweeted delivery).

Paul was a champion at keeping our close friends and family posted on what was happening with Eve in the hospital.  It’s hard to express how grateful I am for this.  He took photos, he blogged. He DID the baby book.  Virtually.  We can still access most of it.  And may go through the process of pulling Facebook and blog posts into a more permanent format, so Eve can read her own story someday, as so many others have told it too.  Some of Paul\’s posts were so profound (still are, even this week)  that I still revisit them now and then for a moment of thankfulness and grounding.

Eve and Paul, Children's HeartLink gala, 2009

We think she’s a pretty cool kid – with a pretty cool story (minus the actual baby book).  For now, the locks of hair, the pulse ox wrap and her ankle bracelets can stay in a keepsake box.  Her life continues to be full of firsts.  The dates don’t really matter.  What matters is that she came to us, she powered through, and she is here to ask her daddy all about it.

Today is not normal.

Today is not normal.

February 7, 2013

Today should have been a celebration of my husband’s birthday.

Today should have been a day to rally around Congenital Heart Awareness Week.

Today should have been the start of our wedding reunion weekend in California.

Today should have been a day to finish up some work, get the kids to school, face the Minnesota winter.

But that’s not today.

Today our 4-year-old daughter Eve, is having neurosurgery – 1500 miles from home – to remove a brain tumor.  Nothing about today is normal.

I do not want to know what a cranial resection entails, no more than I wanted to know what a heart ablation or mitral valve prolapse entailed 4 years ago.

I do not want to understand that my little girl will be awakened mid-surgery, while her skull is still open, to make sure her limbs can move.

I do not want to know her fear and confusion.  I do not want her to be in pain.

I do not want to know that this sweet child, who has not been on this planet even 50 months, has had two heart surgeries and a brain surgery.

I do not want to know.  But I must know.

That is our job, as her parents and protectors.  To be smart and strong.  To understand what we can, and question what we don’t.  No stone can be left unturned.

I have no idea how such misfortune could befall one small girl.  But I do know her scars – on her chest, and now on her sweet head – will not define her.  They are part of her journey now – but her life will have meaning and beauty and power not because of the scars, but in spite of them.  Yes, I hope that somehow these new challenges will make a difference in other lives, as I know her heart has.  That is the only way to see around the randomness of it all.

Until today, we were 1in100.  Now we are also 1in1000.  Childhood brain tumors affect 4,200 kids in the U.S. each year.  Almost the same number of infants – less than one year old – die each year in the U.S. from congenital heart disease.  Comparing statistics is one thing. Comparing merit is quite another.

So, in honor of Eve, this is my small contribution to CHD Awareness Week: to NEVER again compare funding dollars or research numbers between horrific pediatric diseases.  I don’t care how common or downright rare the disease is.  If it’s your child, there is but one answer: make it go away.

Just because pediatric heart disease is more prevalent than pediatric cancer does not make it more important. Comparing research methodologies or means for two wildly different diseases is like comparing a paddleboat to a jetski.

Awareness is nothing without data. Data is nothing without people. People are nothing without collaboration. Collaboration is what makes the information meaningful enough to do something with it. I don’t care if you are a parent, a physician, a researcher or a technician – no one wants to feel like they are bringing a toothpick to a gunfight. Listen to each other. Learn from each other. Share information that can improve chances.

No one wants children to have their chests or skulls cut open to survive.  Even the doctors that do it don’t want to do it. They just have to do it.

Until we can prevent these things from happening in the first place, today we have early detection, access to effective treatment, meaningful data, constantly improving technologies and collaboration.

Today is not normal. Today my daughter becomes both a heart survivor and a brain tumor survivor. And she is amazing.

 

 

Amanda Rose Adams takes it to the hill. Literally. We all have a role to play in advocacy.  She can help educate us all on finding the gumption to do it.  http://www.savebabies.org/blog/2012/06/not-my-fight-now-my-fight/

Health information sharing.

Health information sharing.

Last week’s study published in the New England Journal of Medicine proclaiming patients taking azithromycin (Z-Pack) had an increased risk of cardiovascular death gave me goosebumps.  Again.  Not because I have a father with heart disease and 2 replaced valves.  Not because I have a 3-year-old daughter with Congenital Heart Disease.  But because it reminded me, yet again, that relevant health information is rarely collated into one place and shared effectively to protect the patient.

When my daughter, Eve, was not yet one month old, we were given “training” on how/when to administer our baby’s cocktail of 8 medications. These were the drugs that were keeping her heart beating. Period. No pressure.  Among the 8 meds, was a drug called Digoxin.  It’s a widely used medication for heart conditions – in adults and children – and essentially improves the pumping function of the heart.  But it is also technically incompatible with certain types of heart arrhythmia, and particularly with electrical pathway issues.

If not for a diligent pharmacist, we may have been sent home with a baby with CHD and an undiagnosed case of Wolf-Parkinson-White Syndrome.  Wolff-Parkinson-White syndrome is a heart condition in which there is an extra electrical pathway (circuit) in the heart. The condition can lead to very rapid heart rate, called supraventricular tachycardia (SVT).  In our daughter’s case, it was 250-300 beats per minute.  “Like a hummingbird” our pediatric cardiologist used to say.  Question is: why wasn’t this immediately apparent to every one Eve’s care team?  Why did we just get lucky?

This nearly-adverse event because one of the core elements of change I was committed to working on in health IT. The infrastructure is there. The capacity is there. The need is there. Why can’t these systems just TALK to each other??  My mom used to call it the “knitting circle”…in our small hometown, everybody knew everything.  In real time. 30 years ago.

The new report on Zithromax (or Z-Pack as it’s fondly known) was just another of a thousand case examples where health IT should be solving a problem.

In full disclosure, I love the Z-Pack.  We’re fortunate in that our kids don’t get infections often, but when they do, the other stuff just never works.  Z-Pack does.  And it’s a short course.  Easier to take, easier to complete.  I can think of one instance where all three of the children had nasty cases of some upper respiratory infection.  The pediatrician prescribed Z-Packs.  It was smooth sailing for our older two, but Eve (then 20 months) would not touch it.  We could not get a drop into her, without being charged with some sort of child abuse.  We let it go.  It took her longer to recover, but eventually she did.

Clearly our pediatrician is aware of Eve’s heart condition.  But would she have been aware of the potential issues with azithromycin relative to heart arrhythmias and accessory pathways?  Apparently, there has been an awesome little tool our there for years to help electrophysiologists make antibiotic choices in patients with Long QT syndrome or in those who take other antiarrhythmic drugs.  There’s even the Arizona CERT QTDrugs.org website. But primary care physicians having unfettered access to details that specialists may have at the top of their newsfeed? Well, that’s just not reality. Most of us just hope that an integrated care team is reality.

But those of us who BELIEVE in the power of health IT understand it DOES reduce disparities and improve outcomes. It allows that team, however loosely structured, to actually communicate, exchange health information and share critical data.  It reduces the risk of the wrong prescription. The the wrong diagnosis. The wrong care management.  It’s not just a virtual “red flag” – it’s the knitting circle that both care providers and patients desperately need.

It’s Awareness Day…and??

It’s Awareness Day…and??

February 14  is Congenital Heart Defect Awareness Day.  It’s also the day our 2 month old almost lost her life on the way to a hospital emergency room.  Not a great memory or reason to celebrate.  But is is a reason to share, which is what thousands of families are doing on this day.

When Eve was diagnosed at 48 hours old, the doctors said she had unknown “congenital heart defects” and possible pulmonary hypertension of the newborn, resulting in a diagnosis of Congenital Heart Disease.  What is “congenital”??  Is that like “congestive” heart failure?  Is it a disease?  Or a defect?   Tip: do not Google “pulmonary hypertension of the newborn” from your hospital bed at 2 AM after they have taken your baby away to the ICU.

She would struggle to make it past one week old.  But after she did – through a cocktail of 7 round the clock medications to keep her heart functioning – we started digging into what CHD really meant.  Fact: we had no clue.  And I had been around heart stuff…serving on advisory committees for the American Heart Association and Children’s HeartLink…and had personal friendships with some of the pioneers in cardiac medical devices.  My dad had Rheumatic fever as a child – and had subsequent heart issues.  His mother – my grandmother – was a blue baby, and in the 1950s was among the first patients on Dr. Walt Lillehei’s heart lung machine.  That surgery extended her still abbreviated life by almost 5 years…long enough to see one of her 4 boys graduate high school.

Still, with all this, I was a complete idiot on CHD.  This deer-in-the-headlights response recurs almost daily when we use the term (or the acronym) to explain what happened to our daughter Eve.  People just don’t get it.  Lumping Critical Congenital Heart Disease (CCHD) in there – and seriously, it’s almost time to throw in the towel.

The simplest way around this so far has been this: Eve was born with heart defects that caused her heart disease – which she will have for the rest of her life.

I think what’s unique to the CHD community is that we are dealing with a BIRTH defect.  Explaining that’s what “congenital” means is important, but not required.  I think great strides can be made when we start talking about “heart defects” and “heart disease”.  People CAN get their heads around that.  And they can talk to others about the prevalence, the need for research and the astounding progress that has been made to improve the long-term survival rates.

At 15 and 16 weeks old, Eve would undergo two surgeries to repair her heart defects.  Her recovery after was nothing short of miraculous. Today, she is a boisterous 3-year-old with only the remnants of the scars from her open heart surgery.  1in100.org and facebook.com/1in100 began with the purpose of connecting families and impacting health policies that can improve outcomes for pediatric heart patients.

The first priority was early detection of critical heart problems that kill and compromise babies.  Heart defects are the leading cause of infant death from birth defects, claiming the lives of more than 4,000 babies in the U.S. each year.  Fortunately, a simple, non-invasive test in the newborn nursery can help clinicians detect possible heart problems, ensuring earlier intervention and better outcomes.

It was a two-year crusade.  One that reinforced the utter lack of understanding of what CHDs are and how often they occur.  One percent of newborns are impacted. The population surviving with congenital heart disease is growing 5 percent each year.

This month, I have the sincere honor of presenting hospital Grand Rounds with Eve’s cardiology team in Minnesota, and will be addressing the topic of newborn screening for heart defects at Cardiology 2012, attending by clinicians and medical experts from around the world.  I’m safe with the CHD and CCHD acronyms among these folks.  It’s the rest of the world that needs to understand.  And if kicking the acronyms to the curb works, then let’s roll with it.

Finding Christmas.

Finding Christmas.

Three years ago today we spent Christmas Day at the side of our daughter Eve’s isolette in the PICU at the University of Minnesota Amplatz Children’s Hospital.  She was 13 days old.  It was the first – and likely the only time I will see Santa cry.

People still comment on how horrible that must have been – spending Christmas in the hospital with a sick baby, away from our other children and family.  I don’t remember it that way.  I remember the exponential gratitude of being there, where Eve was being nurtured and cared for by experts. She was struggling, but she was alive.  That’s what mattered.  And it was in that spirit of hope that Christmas remains the most sacred of days for us.

Here’s my wish – today and every day:  that people recognize the blessing of having access to medical teams that can heal broken hearts.  This is reality. In 98 percent of the rest of world, we would have just been another set of grieving parents.  Eve would have never survived for 13 days.  She would have been gone.

To those sitting in those hospital rooms tonight, we wish you hope, grace and comfort.  May your babies come home soon and thrive.  To those who know others sitting in hospital rooms tonight, find it in your hearts and in your hectic schedules to GO THERE.  Be there for those enduring this journey.  We know it’s hard.  Sick babies and children are not the things Christmas dreams are made of.  In fact, it’s just downright wrong. Anytime. Anywhere.  But these families need you.  Show up.  Be present.  Give, if you can.

During that first month, 3 co-workers and less than half a dozen friends visited us there.  I understood why, but that didn’t make it any easier.  Much of the saving grace came from emails, facebook posts and blog entries.  In this community of congenital heart defects, virtual strangers become virtual lifetime friends.  We find out how many 1in100′s there are. Perhaps that is the gift of adversity.  And it is an amazing thing to behold.

The Birth Announcement (That We Didn’t Want to Send)

The Birth Announcement (That We Didn’t Want to Send)

Eve Isley Saarinen was born the evening of Friday, December 12th  – 6 lbs, 9 oz, 20 inches… lots of beautiful dark hair.

At 2 days old, Eve was diagnosed with congenital heart disease. It’s a broad category.  Her biggest problem is a severely leaky mitral valve, which puts strain on the whole heart function, enlarging the heart and putting pressure and excess fluid on the respiratory system.  She was transported by Lifelink to the University of Minnesota NICU on the 14th.  Paul drove through the blizzard that night to be at her side, but since I had a c-section I could not be discharged until the next day.  Took a cab over there on the 15th and we hunkered down for the next 5 days.  The medications seemed to be working at first, but within a couple days her heart was continuing to get larger, her breathing more labored.  After an episode of SVT – which is basically super-fast heart arrhythmia, she was immediately transported to the U of M Children’s Hospital pediatric intensive care unit – closer to the full time cardiology staff and cardiac surgeon.

She was started on a new, stronger drug to alleviate some of the pressure and workload on her heart.  We were warned that her situation was precarious.  The surgeon and team spoke to us of a possible transplant (her heart had already been greatly damaged by her defect and the tachycardia).  Yet with all this, she has started to turn a corner and improve.

It is slow progress – and it’s hard to be patient.   We don’t know how long we’ll be here as the medicines do their work…though they seem to be stabilizing her.  We’ve told our parents to come now…want them to know her if God takes her from us now.  Just writing this is almost unbearable.  For now, we are only positive around her…so we cling to a tentative plan to get her bigger and stronger for a heart surgery at about 3 months old.

There are very sick children all around us here.  It’s the holiday season and there shouldn’t be any sick children.  Anywhere.  So we pray for all these babies and continue to be grateful for how well Eve has been doing.

Blessings to all of you in the new year – cherish your new arrivals.  They are the greatest gifts.  We’ll do our best to keep you posted (Paul’s Eve blog updates can be found at:  www.tweetandmeet.com)

Annamarie, Paul, Jack, Elle and baby Eve

Consensus – AAP, AHA, ACC Endorse Screening Newborns for Heart Defects

Consensus – AAP, AHA, ACC Endorse Screening Newborns for Heart Defects

Published in the journal Pediatrics: Endorsed by the American Academy of Pediatrics, the American Heart Association and the American College of Cardiology, a federal advisory panel recommends nationwide screening for critical congenital heart disease using pulse oximetry, a probe placed on a hand and a foot that uses a light source and sensor to measure oxygen in the blood. Low oxygen levels signal the need for further testing to look for a heart-related problem.

View the report here: Newborn Screening for Heart Defects Using Pulse Oximetry Recommended

There are few words to describe the excitement.  Thinking back to Eve’s Impact, her diagnosis and surgeries – and on to that day in the fall of 2009, when the team in Minnesota agreed to pursue a pilot project, to discussing a federal nomination with the committee member, to providing comments to the federal advisory committee and participating in this federal workgroup that yielded this report..it’s been a whirlwind.  Now, all this news coverage in the past 24 hours.  It’s clear the time has come to use all the tools available to support the early detection of heart defects in newborns.

http://www.cbsnews.com/video/watch/?id=7377689n&tag=mg;earlyshow

http://articles.latimes.com/2011/aug/21/health/la-he-heart-screening-20110822

http://www.webmd.com/parenting/baby/news/20110819/blood-oxygen-test-finds-heart-defects-in-newborns

http://www.medscape.com/viewarticle/748382

http://www.baltimoresun.com/health/boostershots/la-he-heart-screening-20110822,0,1119997.story

http://seattletimes.nwsource.com/html/health/2015978538_webheart22.html

http://www.smartplanet.com/blog/rethinking-healthcare/could-a-new-screening-help-identify-heart-problems-for-newborns/6335

http://health.usnews.com/health-news/family-health/heart/articles/2011/08/22/steps-outlined-to-screen-all-newborns-for-heart-defects

http://blogs.wsj.com/health/2011/08/22/screening-newborns-for-congenital-heart-disease/?mod=WSJBlog

Early Detection of CCHD – Pulse Oximetry Advocacy

Early Detection of CCHD – Pulse Oximetry Advocacy

Pulse Oximetry Advocacy Temporary Toolkit:

Interactive screening map with current state legislation, legislation pending and hospitals screening for CCHD:

http://www.cchdscreeningmap.com/

I. INITIAL OUTREACH TARGETS

State Departments of Health

Newborn Screening and/or birth defect surveillance divisions

Hospital Medical Staff – contacts

Pediatric Cardiology
Neonatology
Pediatrics
Nursing
Newborn nurseries/Labor & Delivery

Hospital Administration

CEO
Chief Financial Officer
Medical Director
Nursing Executive Leadership
Patient Safety/Patient Care

Organizations

AAP – State Chapter leadership  – AAP Chapters

Hospital Associations by State

America Heart Association (state chapter)

Pediatric Heart Organizations /Sites (some with State Chapters)

Mended Little Hearts
It’s My Heart
Lasting Imprint
Congenital Heart Information Network
Bless Her Heart
Children’s Heart Foundation
James’ Project
CHD Speaks
BabyCenter Community: Babies and Children with Heart Problems
Helping Hands, Healing Hearts
CHD Babies
II. INITIAL TALKING POINTS

Website and address of every hospital in the US, by state: http://hospitalandmedicalcentercompare.com/by-state

Draft Hospital Outreach Letter

Click here for the Word document file.Letters sent in support of Newborn Screening for CCHD to the Department of Health and Human Services

Children’s National Medical Center, Washington DC

Darshak Sanghavi, M.D., UMass Memorial and University of Massachusetts Medical School Letter

Patient Safety Advocates Letter

 

III. FEDERAL RECOMMENDATION – NEWBORN SCREENING FOR CRITICAL CONGENITAL HEART DISEASE (using Pulse Oximetry)

On October 15, the Secretary’s Advisory Committee on Heritable Disorders in Newborns and Children sent a letter to Secretary Sebelius recommending all newborns be screened for Critical Congenital Heart Disease using pulse oximetry.  The Secretary responded on April 21, 2011, by asking the newly convened Interagency Coordinating Committee (ICC) to review the recommendation and specifically address implementation and infrastructure gaps associated with state by state adoption of this screening.  The ICC has been tasked with providing a full action report within 90 days (which would be before the third week of July, 2011 or sooner).
Secretary Sebelius
The interim period is still an excellent time to weigh in with Secretary Sebelius in support of early detection of heart defects.  Advocates may use their own experience, share a screening update from their state or advocate broadly for national screening.
To reach the Secretary of Health and Human Services, call 202-690-7000.  After hours, there is VM comment line: 202-205-5445 

Or email your personalized letter/note to the Secretary at: Kathleen.Sebelius@hhs.gov

You can copy and paste your letter right into the email.  Use the subject line:  “Support Newborn Screening for CCHD”

Congress
Advocates may also weigh in with the Congressional Members in support of saving lives through screening for CCHD.

Use this link to find your delegation, with emails and phone numbers:

http://www.contactingthecongress.org/

Sample Letters

Use the following sample letters for talking points – or customize however you’d like.  The more personal the better.

Sample letters – one for Secretary Sebelius: Family letter_HHS_NBS CCHD

and one for a Congressional member:  Family letter_Congress_NBS CCHD

Here’s the background briefing we’ve been using with policy leadership as well.

Outreach to State Elected Officials/Government

Directory of Federal, State, Local Officials and Government Agencies

SHARE

The online CHD community is powerful.  Utilize the following resources to mobilize, gather and share information:

Cora’s Hopes and Dreams

Children\’s National Medical Center Pulse Ox Program

Facebook Pages:

Pulse Oximetry

Pulse Ox Please

Check Their Hearts: Support Pulse Oximetry Screening for Heart Defects

Pulse Ox Pennsylvania

Pulse Ox Mississippi

CHD Babies

1in100

 

 

Up Next: Newborn Screening for Heart Defects

Up Next: Newborn Screening for Heart Defects

The Department of Health and Human Services just held a stakeholder call to update on the status of the recommendation to screening newborns for CCHD with pulse oximetry. Given the 5 recommendations related to this screening, the Secretary has found several items she would like to have further clarification on to move forward with the screening (see the original recommendation letter here). 

It was clearly stated that the Secretary recognizes the critical importance of CCHD, and has requested further efforts be undertaken immediately to work through the issues surrounding the pulse ox technology itself, follow up diagnostics, service infrastructure, and education. The Newborn Screening Saves Lives Act (of 2007) required the formation of a supplemental Interagency Coordinating Committee on Newborn and Child Screening issues. This committee includes directors of the CDD, HRSA, NIH and AHRQ. The committee has been IMMEDIATELY tasked with reviewing the original SACHDNC five recommendations, any gap areas, and the document produced from the Implementation Workgroup – their goal is to close the gaps. It is the committee’s first order of business…and they have 90 DAYS to provide a plan of action.

Here’s what I know. Without the outreach all of you did, sharing your support and personal stories, this important secondary advisory committee step wouldn’t be happening. This was going down the road of being an averted recommendation. Now it’s not.

So while we might not have the green light quite yet, the motor is running and the car is in gear. Please accept my profound gratitude for all you continue to do to support early diagnosis of heart defects.

I can be honest here, with all of you. These last 10 days have been hard. a hundred hours of time, anxiety, nausea, all of it. My first reaction to the 90 day delay was this: in 90 days, 1,000 more babies will die – and realistically, 200-300 of them will be contributed to by late diagnosis.

But I also realize there is solid intent on the part of the federal stakeholders to get this right. In that spirit,  let’s support this effort, continue the work already being done, and look forward to widespread implementation of newborn screening for CCHD.   #1in100